ABSTRACT
Resumen La enfermedad de Whipple es una enfermedad multisistémica crónica, causada por la bacteria Tropherima whipplei. Se han descripto aproximadamente 1200 casos en la literatura. La incidencia mundial se estima en 9.8 casos por millón de personas. Los datos provenientes de Sudamérica y Europa muestran que afecta a varones de mediana edad. Se cree que los factores inmunológicos del huésped son los que influyen en el curso de la infección y no el genotipo del agente. Dado que las características clínicas no suelen ser específicas y el espectro de manifestaciones en órganos individuales puede ser subestimado, el diagnóstico sigue siendo muy difícil. Presentamos un caso confirmado por histopatología con compromiso multisistémico. Consideramos importante su difusión dados los escasos casos documentados en Sudamérica y la relevancia de tener presente la sospecha diagnóstica para el abordaje terapéutico precoz que mejora el pronóstico de esta rara enfermedad.
Abstract Whipple's disease is a chronic mutisystem disease caused by the bacteria Tropherima whipplei. Approximately 1200 cases have been described in the literature. The worldwide incidence is estimated at 9.8 cases per million people. Data from South America and Europe show that it affects middle-aged males. It is believed that host immunological factors rather than agent genotypic traits influence the course of the infection. Since the clinical characteristics are usually nonspecific and the wide spectrum of manifestations in individual organs may be underestimated, the diagnosis remains challenging. We present a case with multisystem compromise confirmed by histopathology. We consider its publication important given the few cases documented in South America and the relevance of bearing in mind the importance of an early diagnosis for a prompt treatment that improves the prognosis of this rare disease.
Subject(s)
Humans , Male , Middle Aged , Aged , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Whipple Disease/complications , Whipple Disease/diagnosis , Whipple Disease/drug therapy , Europe , Tropheryma , Anti-Bacterial Agents/therapeutic useABSTRACT
Whipple's disease is a chronic mutisystem disease caused by the bacteria Tropherima whipplei. Approximately 1200 cases have been described in the literature. The worldwide incidence is estimated at 9.8 cases per million people. Data from South America and Europe show that it affects middle-aged males. It is believed that host immunological factors rather than agent genotypic traits influence the course of the infection. Since the clinical characteristics are usually nonspecific and the wide spectrum of manifestations in individual organs may be underestimated, the diagnosis remains challenging. We present a case with multisystem compromise confirmed by histopathology. We consider its publication important given the few cases documented in South America and the relevance of bearing in mind the importance of an early diagnosis for a prompt treatment that improves the prognosis of this rare disease.
La enfermedad de Whipple es una enfermedad multisistémica crónica, causada por la bacteria Tropherima whipplei. Se han descripto aproximadamente 1200 casos en la literatura. La incidencia mundial se estima en 9.8 casos por millón de personas. Los datos provenientes de Sudamérica y Europa muestran que afecta a varones de mediana edad. Se cree que los factores inmunológicos del huésped son los que influyen en el curso de la infección y no el genotipo del agente. Dado que las características clínicas no suelen ser específicas y el espectro de manifestaciones en órganos individuales puede ser subestimado, el diagnóstico sigue siendo muy difícil. Presentamos un caso confirmado por histopatología con compromiso multisistémico. Consideramos importante su difusión dados los escasos casos documentados en Sudamérica y la relevancia de tener presente la sospecha diagnóstica para el abordaje terapéutico precoz que mejora el pronóstico de esta rara enfermedad.
Subject(s)
Hypertension, Pulmonary , Whipple Disease , Aged , Anti-Bacterial Agents/therapeutic use , Europe , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Male , Middle Aged , Tropheryma , Whipple Disease/complications , Whipple Disease/diagnosis , Whipple Disease/drug therapyABSTRACT
Whipple's Disease, a rare diagnosis caused by the slow-growing bacterium Tropheryma whipplei, most often presents with the classically described signs of malabsorption due to gastrointestinal colonization. However, it can also have signs and symptoms that clinically overlap with rheumatic diseases, potentially resulting in misdiagnosis. Furthermore, treatment with modern potent biologic immunosuppressive agents and classic disease modifying anti-rheumatic drugs (DMARDs) can lead to serious exacerbation of undiagnosed infections. We present the case of a middle-aged woman with long term complaints of arthalgias, who was diagnosed with seronegative rheumatoid arthritis and subsequently treated for almost 7 years with such immunosuppressive therapies. The patient's disease course included chronic diarrhea that abruptly intensified and culminated in fatal hypovolemic shock/sepsis. A diagnosis of WD was made by autopsy examination, wherein several organ systems were found to be heavily involved by Tropheryma whipplei organisms, and their identification was confirmed with histochemical and molecular evaluation. Notably, most bacterial organisms were located deeply in the submucosa/muscularis of affected organs, a practical reminder to practicing pathologists that challenges the classic histopathologic description of Whipple disease as an infiltration of predominantly lamina propria, and the potential for sampling bias in typically superficial endoscopic biopsies during routine procedures.
Subject(s)
Humans , Female , Middle Aged , Actinomycetales Infections/pathology , Tropheryma , Whipple Disease/complications , Whipple Disease/pathology , Autopsy , Rheumatic Diseases/complications , Sepsis/etiology , Diagnostic Errors/prevention & controlABSTRACT
BACKGROUND: Whipple's disease is a rare systemic disease caused by a gram-positive bacillus called Tropheryma whipplei. First described in 1907 as an intestinal lipodystrophy with histological finding of vacuoles in the macrophages of the intestinal mucous. Usually the symptoms are localized according to the compromised organ. The differential diagnosis is wide. It can be fatal without proper treatment. Recurrence can occur in up to 33% of the cases and usually compromises the neurological system. CASE PRESENTATION: This article reports the case of a 46-year-old female patient with a history of a 6-month hypochromic microcytic anemia of unknown cause. She consulted for a 6-months oppressive abdominal pain located in the mesogastrium as well as abdominal distention associated with nausea and liquid stools; in addition, she had an 8-month small and medium joint pain, without edema or erythema. Physical examination without relevant findings. Multiple esophagogastroduodenoscopies with normal gastric and duodenal biopsies findings and a normal colonoscopy were performed. Endoscope capsule showed red spots in the duodenum and ulcerations in the jejunum and proximal ileum covered by fibrin; histological report showed macrophages with positive periodic acid-schiff reaction staining (PAS staining), disgnosing Whipple's disease. Antibiotics were initiated. The patient is currently in the second phase of treatment without gastrointestinal and joint symptoms. CONCLUSION: This is the first case reported in Colombia. It is a rare entity and difficult to diagnose reason why it is important to continue with clinical investigations to give more clarity about the onset and appropriate diagnose to avoid the delay in treatment of this entity.
Subject(s)
Whipple Disease , Anti-Bacterial Agents/therapeutic use , Colombia , Endoscopy, Gastrointestinal , Female , Humans , Middle Aged , Tropheryma , Whipple Disease/diagnosis , Whipple Disease/drug therapyABSTRACT
Whipple's disease is a rare disease caused by the actinomycete bacteria Tropheryma whipplei, which cause intestinal infection. The most common symptoms are chronic diarrhoea, weight loss, abdominal pain, arthritis and neurological abnormalities, which can be fatal. This paper reports a case of a 57-year-old Brazilian woman with diarrhoea, vomiting, abdominal pain, appetite loss, intermittent fever, malaise, weight loss and malnutrition. Migratory polyarthralgia and recurrent visual scotomas preceded the symptoms. The retroperitoneal pseudotumour formation finding was associated with prolonged wasting syndrome, which did not respond to usual therapies, thus leading to the investigation of carcinomatosis disease. After laparotomy, biopsy and histochemical study of the lesions with negative results for malignancy, we proceeded to the investigation of Whipple's disease, which was then confirmed. The patient improved clinically and started gaining weight after using ceftriaxone (IV).
Subject(s)
Female , Humans , Middle Aged , Carcinoma/diagnosis , Tropheryma/isolation & purification , Whipple Disease/diagnosis , Anti-Bacterial Agents/therapeutic use , Ceftriaxone/therapeutic use , Diagnosis, Differential , Whipple Disease/drug therapyABSTRACT
Whipple's disease is a rare disease caused by the actinomycete bacteria Tropheryma whipplei, which cause intestinal infection. The most common symptoms are chronic diarrhoea, weight loss, abdominal pain, arthritis and neurological abnormalities, which can be fatal. This paper reports a case of a 57-year-old Brazilian woman with diarrhoea, vomiting, abdominal pain, appetite loss, intermittent fever, malaise, weight loss and malnutrition. Migratory polyarthralgia and recurrent visual scotomas preceded the symptoms. The retroperitoneal pseudotumour formation finding was associated with prolonged wasting syndrome, which did not respond to usual therapies, thus leading to the investigation of carcinomatosis disease. After laparotomy, biopsy and histochemical study of the lesions with negative results for malignancy, we proceeded to the investigation of Whipple's disease, which was then confirmed. The patient improved clinically and started gaining weight after using ceftriaxone (IV).
Subject(s)
Carcinoma/diagnosis , Tropheryma/isolation & purification , Whipple Disease/diagnosis , Anti-Bacterial Agents/therapeutic use , Ceftriaxone/therapeutic use , Diagnosis, Differential , Female , Humans , Middle Aged , Whipple Disease/drug therapyABSTRACT
Whipple's disease is an infrequent multisystemic infection caused by a gram-positive bacterium: Tropheryma whippelii, which after several studies has been characterized as an actinomiceto por 16Sr RNA. It occurs with multiple symptoms, the principal of which are diarrhea, weight loss, stomach pain and arthralgias. Arthritis or artralgia may appear as an isolated symptom and eventually through the years additional digestive, cardiovascular and/or neurological symptoms arise. Diverse immunological abnormalities usually present before or after clinical symptoms are first discovered. Currently there are cabinet, endoscopic, radiological, tomographic and laboratory studies which can help to make a definitive diagnosis, such as the duodenal biopsy submitted to the Schiff test, to the polimerasa chain or an electronic microscopy in order to see the intracellular bacteria in the macrophage and for immunohistochemistry to see specific antibodies to Whipple's disease. Treatment is trimetoprim/sulfametoxazol, it is suggested transfer factor too.
Subject(s)
Whipple Disease , Anti-Bacterial Agents/therapeutic use , Antibodies, Bacterial/blood , Arthralgia/etiology , Diarrhea/etiology , Duodenum/immunology , Duodenum/microbiology , Humans , Macrophages/microbiology , Transfer Factor/therapeutic use , Trimethoprim, Sulfamethoxazole Drug Combination/therapeutic use , Tropheryma/genetics , Tropheryma/immunology , Tropheryma/isolation & purification , Weight Loss , Whipple Disease/diagnosis , Whipple Disease/drug therapy , Whipple Disease/immunology , Whipple Disease/microbiology , Whipple Disease/pathologyABSTRACT
Os autores relatam um caso de Doença de Whipple em paciente do sexo masculino, de 55 anos, que apresentava predominância de sintomas gastrintestinais e importante comprometimento do estado geral, caquexia e anemia. O diagnóstico foi confirmado por exame histopatológico. Após o tratamento com antibióticos, observou-se excelente evolução clínica. Salientam-se a importância e a atualidade do diagnóstico, visto que, apesar de doença pouco frequente, é potencialmente fatal e responde drasticamente ao tratamento com antibióticos.